Monday, December 13, 2010

Mind Blown

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A new diagnosis.
AND spaghetti O's

It's been quite a day here in Shimmy La Land.

I'll start with the spaghetti o's. The day began with a lighting bolt of an idea...ok, ok!  Fine, I'll start with the diagnosis one.  That's the one that's inspired the title of this blog anyway.

Simon and I were at Children's Hospital today to celebrate our dear friend Saun-Toy. She was receiving her Employee of the Month award and there was cake.

As we're headed to the elevator on the 2nd floor (post cake and Simon saying "Mazel Tov" to all the recipients- during the CEO's speech) we pass the door to the Cardiology offices.

Simon makes a beeline for the reception desk to say his hello's. Of course we ask if Dr HunkyPants is around, just to say Hi.

He is and while we're exchanges pleasantries/exclamations of how good Simon looks (us/him) he mentions in passing that he finally got Dr. Towbin on the phone and had a conversation with him about Simon.

This is Dr Towbin who is the foremost cardiologist in the country for Pediatric Cardiomyopathy. The Dr that families from all over the country if not the world come to see when it comes to this frikkin' disease and making sure that as much of it can be understood as possible. We've been trying for months to get him and his office to return our calls and have sent in Simon's records twice to see if we can get him to simply look at them and make sure that we're on the right path.

Quick shout out to Dr Rosenfeld for being the type of doctor that has no problem working in the best interest of the child and consulting left and right both when he and we have thought it might benefit Simon. Now that's an awesome quality to have in your primary cardiologist!!

Hunkypants continues to say that while Towbin was looking at Simon's echocardiograms, he seemed to think that there was in fact evidence of non compaction of the left ventricle. What this means is that Simon's Dilated  Cardiomyopathy (DCM-a very large umbrella term) has an additional diagnosis of LVNC (left ventricle non compaction) that allows us two things: 1) a greater chance at understanding where his DCM may have come from (70% of patients with LVNC can be traced back to a genetic disorder and there's a test for it)
2) give us greater insight into a longer term prognosis.

Now while #1 is nice and all, since it won't change the management of Simon's heart all that much, I'm interested to see if our insurance will pay for the genetic testing but won't cry buckets if it won't.
 I'd be happier to not have to say Simon's condition is idiopathic but it's not going to rock my world.

What does rock my world though is hearing Simon's Dr say "what's good though is that we know patients with LVNC tend to be less symptomatic and do better with their decreased function than those kids with just DCM." It means that there's greater understanding of this particular subset of people living with DCM. It's more information on how the heart is working or 'not' working as the case may be. LVNC is about the makeup of the muscle and not just it's function.
The muscle itself is spongy or not-compacted- see the channels in the left ventricular bottom wall..

It's a lot more than you need to think about dear reader. However, what it means that I get to think about is more information, greater detail in terms of prognosis (and even better that it's a more positive prognosis), and subtle but distinct steps to take in maximizing Simon's treatment.


We will move forward with making sure that Simon is at the maximum doses for all his medication, especially his beta blocker Carvedilol, and look closely in future echo's at the structure of the left ventricle.

It's not like we've discovered anything monstrous but gosh darn it, it feels huge. So much of what is so amazingly difficult in this world of Cardiomyopathy are the unknowns.
Where it came from,
what tomorrow might be like,
what five years from now might be like,
never trusting in the improvements completely but giving thanks for each season spent out of the ICU,
never ever getting to say it's 'resolved,
seeing the wide spectrum of what it means to 'live' with this disease (playing organized sports to being wheelchair bound with 24/7 oxygen),
and waiting, always waiting to hear that the heart function has increased or decreased after every Echo knowing that it really could go either way no matter how long you've been at this.

It's just a little more clarity. Like knowing that the waters that you're swimming in are in fact salt water and a little more buoyant than you previously thought.

 I still feel the vastness of it, I'm still swimming with no sign of land, but I can understand my environment even just a smidge better and rely somewhat on what I know about swimming in salt water versus freshwater.

Speaking of saltwater- amazing transition Laura!- Simon has a new found love.

The short version is that Simon is eating!! Something has shifted in the last two weeks and all of a sudden we are on the fast track to getting Simon eating orally versus being tube fed. This morning we went out to Trader Joe's and bought Simon his first can of Joe's O's (essentially Spaghetti O's) and gosh darn it, if the boy didn't take at least 10 licks off a spoon, including some biting and chewing of the smaller o's. I nearly crapped my pants.

We sit down and play with food no less than five times a day (and by sit down I sometimes mean in the car gnawing on a piece of turkey jerky). But hot damn, to see my son nibble, chew and swallow....and all in the same minute....that's amazing. We don't think about it, and when I say we, I mean you all out there that aren't tube fed, but the simple act of eating....well, it's not.
Simple that is.

And, besides taking the spoon and feeding himself, he also let me, over and over again, get an 'o'  and some sauce in there.
I love getting to feed my son. Just as most parents are thrilled to let their kid's feed themselves, I am getting the experience that I missed out on for the last 2 years. I'm getting to feed my child.

There's that simple lean in that he does when I have the spoon right in front of him that makes my own heart pop a little.

It means not only that he wants what I have to offer to him (and how sweet is it to be able to fullfil a simple desire- how much longer will that last?!) but there is a beautiful element of trust to it.

Simon has not eaten by mouth for over two years. There were a lot of important reasons not to. Staying alive and conserving energy, not wanting to add to the feeling of nausea or bring on vomiting, and last but not least, not having the skills to do it with the consequences being dire (not being able to breathe/ choking).

That he is now allowing me to put food in there (and I mean in there, back on the molars to practice chewing!) I feel so completely honored and grateful.

We've come a long way baby! (reclaiming that phrase).
I know we have a long way to go. We're not making reservations at Chez Panisse anytime soon but I will say that tonight at Sushi, Simon slurped no less than a 1/4 cup of miso soup and gnawed on an almond sized piece of barbequed eel.

That's my boy!!
Slurping Spaghetti O's

Rockin' the Preppy look

One day, one Spaghetti O at a time.